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Open Access 01-12-2020 | Idiopathic Pulmonary Fibrosis | Review

Pulmonary hypertension secondary to pulmonary fibrosis: clinical data, histopathology and molecular insights

Authors: Grégoire Ruffenach, Jason Hong, Mylène Vaillancourt, Lejla Medzikovic, Mansoureh Eghbali

Published in: Respiratory Research | Issue 1/2020

Abstract

Pulmonary hypertension (PH) developing secondarily in pulmonary fibrosis (PF) patients (PF-PH) is a frequent co-morbidity. The high prevalence of PH in PF patients is very concerning since the presence of PH is a strong predictor of mortality in PF patients. Until recently, PH was thought to arise solely from fibrotic destruction of the lung parenchyma, leading to hypoxic vasoconstriction and loss of vascular bed density. Thus, potential cellular and molecular dysregulation of vascular remodeling as a driver of PF-PH has been under-investigated. The recent demonstrations that there is no correlation between the severity of the fibrosis and development of PH, along with the finding that significant vascular histological and molecular differences exist between patients with and without PH have shifted the etiological paradigm of PF-PH. This review aims to provide a comprehensive translational overview of PH in PF patients from clinical diagnosis and outcome to the latest understanding of the histology and molecular pathophysiology of PF-PH.

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Title: Pulmonary hypertension secondary to pulmonary fibrosis: clinical data, histopathology and molecular insights
Authors: Grégoire Ruffenach
Jason Hong
Mylène Vaillancourt
Lejla Medzikovic
Mansoureh Eghbali
Publication date: 01-12-2020
Publisher: BioMed Central
Keywords: Idiopathic Pulmonary Fibrosis
Transthoracic Echocardiography
Pulmonary Hypertension
Pulmonary Hypertension
Pulmonary Hypertension
Pulmonary Hypertension
Published in: Respiratory Research / Issue 1/2020
Electronic ISSN: 1465-993X
DOI: https://doi.org/10.1186/s12931-020-01570-2

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